BACKGROUND Neuroendocrine tumours of the gastroenteropancreatic system (GEP-NETs) are rare, in particular those of the gallbladder. Due to the limited therapeutic options, surgical resection is favoured. METHODS Described below is the case of a 69-year-old male with a lymphogenically metastasising, poorly differentiated neuroendocrine carcinoma of the gallbladder who presented with unspecific abdominal pain. Following complete surgical resection of the tumour and the lymph node metastases he developed a recurrence 6  weeks post-operatively. The recurrence was treated with chemotherapy. Re-staging after three courses, however, showed further tumour progression. Prior to the start of a second-line treatment the patient died 13  weeks after surgery. CONCLUSIONS This case demonstrates the complexity of this rare disease with diagnosis in advanced tumour stage and poor prognosis.